Recent advances on cutaneous lymphoma epidemiology.

BACKGROUND: Primary cutaneous lymphomas are a group of T- (CTCL) and B-cell (CBCL) malignancies. These diseases have different clinical presentations and prognosis. Our knowledge on their epidemiology is limited. Aim of this review was to summarize recent findings on the incidence of CTCL and CBCL, how they change over time, and to describe possible causes and consequences. We found that although there are important differences in the epidemiology of cutaneous lymphomas in different countries, the relative frequency of certain, especially rare lymphomas remains stable. Several studies described growing incidences of both CTCL and CBCL. The emergence of new diagnostic criteria, a more precise definition of the entities and new biomarkers enable a better classification of cases.

[Relevance of plasma exchange in the treatment of myasthenia gravis: study of 11 cases]. / Apport des échanges plasmatiques dans le traitement de la myasthénie: étude de 11 cas.

BACKGROUND: The use of plasma exchange (PE) constituted an advance in the treatment of myasthenia. The objective of our study was to determine the relevance of PE in the treatment of myasthenia and to study the different complications which can be observed during PE. PATIENTS AND METHODS: We studied retrospectively 11 patients who have generalized myasthenia and underwent PE. We used an intermittent flow cell separator and we performed PE three times a week. Biological assessment was performed before and after PE for all patients. The exchange volume was calculated according to the patient weight, gender and the value of hematocrit. RESULTS: Our series included six women and five men. The mean age at onset of the disease was 41.4+/-14.1 years (range: 18 to 68). Indication of PE was myasthenia crisis (eight cases), resistance to classic treatment (two cases) and exacerbation after thymectomy (one case). An improvement was observed rapidly in five cases and delayed in three cases. The remaining three patients did not improve. The most frequent side effects of PE were hypotension (four cases), heart arrhythmia (two cases) and hypoglycemia (one case). Three patients dead in the seven days after the first PE. CONCLUSION: PE represents an interesting tool to treat severe forms of myasthenia and improve prognosis. High incidence of complications in our series can be explained by the initial disease severity, the used method of PE, the existence of associated illness, and a long stay in intensive care unit.

Polysaccharide storage myopathy--case report and literature review.

Polysaccharide myopathy is a rare form of storage muscular disorder. The clinical picture of this particular form of myopathy is unspecific. We report a 62-year-old woman with late-onset progressive weakness and wasting, affecting proximal muscles of the four limbs and the girdles. No myalgia, dysphagia nor symptoms of cardiac failure were observed. Muscle biopsy revealed a vacuolar myopathy with accumulation of amylopectin-like polysaccharide. This material was strongly PAS-positive and diastase-resistant. At electron microscopy, the deposits were composed of non-membrane-bound filamentous and granular material surrounded by numerous mitochondria. No enzyme deficiency was found. Clinical presentation of our patient was similar to the 16 cases reported in the literature. She did not have myocardiopathy and her survival is much longer. Hypothetic mechanisms of polysaccharide accumulation are reviewed.

[Extra-adrenal pheochromocytoma: report of 4 cases]. / Les localisations extrasurrénaliennes du phéochromocytome: à propos de quatre cas.

Extra-adrenal pheochromocytoma is rare. The diagnosis must be evoked in signs of pheochromocytoma with absence of tumor of the adrenal. We report four cases of ectopic pheochromocytoma while specifying their clinical particularities and diagnosis procedure.

[Therapeutic aspects of renal abscess. Report of 50 cases]. / Aspects thérapeutiques des abcès du rein. A propos de 50 cas.

INTRODUCTION: Renal abscess often raises therapeutic problems. Based on their experience and a review of the literature, the authors propose a treatment plant for renal abscess. MATERIAL AND METHODS: The authors report 50 cases of renal abscess treated between January 1988 and September 1999 in the Department of Urology of Charles Nicolle Hospital in Tunis. The diameter of the abscess was less than or equal to 4 cm in 19 cases, between 4 and 10 cm in 29 cases and greater than 10 cm in 2 cases. Renal ultrasound and intravenous urography are an integral part of the initial morphological assessment. All patients were treated by intravenous antibiotics for a mean duration of 28 days. Complementary percutaneous drainage of the abscess was indicated in 25 patients. Surgical treatment was indicated in 13 patients in the presence of a large perirenal collection or severe sepsis, or following failure of percutaneous drainage. RESULTS: Antibiotics alone were proposed in only 17 patients with an abscess less than 4 cm in diameter and 90% of these patients were cured. Percutaneous treatment was performed in 25 patients with an 80% success rate; the other patients underwent open surgery. One patient died after surgery in a context of septic shock despite salvage nephrectomy. The other patients has a favourable outcome. CONCLUSION: The treatment of renal abscess is based on antibiotics alone or combined with a percutaneous or surgical drainage procedure depending on the size of the abscess and the clinical course. The authors describe their decisional flow-chart.

[Cystic cervicofacial lymphangioma]. / Lymphangiomes kystiques cervico-faciaux.

A review of fifteen new cases of cystic cervicofacial lymphangiomas is presented. While discussing the different localizations of these malformations, the authors emphasize the relationships existing between this type of malformation and both the facial nerve and the vasculonervous axis of the neck. They recommend surgical management. 1-to-10 years' follow-up revealed one case of local recurrence associated with incomplete excision.

[Spondyloepiphyseal dysplasia tarda with progressive arthropathy. Apropos of 3 cases]. / Dysplasie spondylo-épiphysaire tardive avec arthropathies progressives. A propos de trois observations.

Two brothers and a sister with spondyloepiphyseal dysplasia tarda who subsequently developed severe generalised peripheral arthropathy are described. The condition appeared to be transmitted as an autosomal recessive trait.

[Genetic study of eight cases of spondylo-epiphyseal dysplasia tarda and its autosomal recessive transmission]. / Etude génétique de huit observations de dysplasie spondylo-épiphysaire d'apparition tardive et de transmission autosomique récessive.

Spondylo-epiphyseal dysplasia tarda is an hereditary disease of bone first described by Maroteaux in 1957. The disease affects only boys, then is transmitted on X-linked recessive pattern. The authors describe two tunisians families with 8 patients presenting clinical and radiologic symptoms resembling spondylo-epiphyseal dysplasia tarda but affecting both sexes. Autosomal recessive inheritance is probable in these families. Conclusion is that genetic heterogeneity of the disease must be precise.